Primary Non-Hodgkin's Lymphoma of the Trachea

Abstract

A 41-year-old woman developed progressive dyspnea during a 3-month period. She was initially diagnosed with asthma and treated medically without significant relief. Chest x-ray was unremarkable. She reported feeling like her “throat was closing up.” Pulmonary function demonstrated a severe obstructive pattern. Her dyspnea continued to progress and eventually the patient reportedly became apneic at home and required resuscitation. A computed tomography scan demonstrated a near obstructing lesion of the distal trachea (Figure 1). She was emergently transferred to our institution. Bronchoscopy revealed a near obstructing polypoid mass 7 cm from the vocal cords (Figure 2). The lesion was detached from the tracheal wall with a snare cautery. The mass was located 3 cm proximal to the carina and 7 cm distal to the vocal cords. The mass measured 2 1.8 1.3 cm and frozen section analysis revealed a poorly differentiated carcinoma. Five days later, she underwent a sternotomy with resection of 2 cm of trachea, 3 cm proximal to the carina. Subsequently, final pathology demonstrated an anaplastic lymphoma kinase positive large B-cell lymphoma. Resection margins were free of tumor. Immunohistochemistry was positive for anaplastic lymphoma kinase-1, CD138, and MUM1. Ebstein-Barr encoded RNA was negative as was CD30 and CD20. One paratracheal lymph node was involved out of 17 sampled. Tumor was stage II by the Ann Arbor staging system. Six cycles of cyclophosphamide, doxorubicin, vincristine, and prednisolone chemotherapy were administered postoperatively. At 4-month follow-up, the patient is alive and well without clinical or radiographic evidence of recurrent disease. Primary tumors of the trachea represent about 0.1% of all malignancies.1 Primary non-Hodgkin’s lymphoma of the trachea is even more uncommon, representing 0.23% of all tracheal tumors.2 The most comprehensive review of the literature identified only 28 primary tracheal lymphomas from 1973 to 2000.3 Median age of presentation was 44 years